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guardians of children with brain or spinal cord tumours
Al54
Abstracts
Section XIX: Turnout-s 281 Isolated extrapontine myelinolysis for craniopharyngioma
following
surgery
J C CUVELLIER,’ C DE SiZE,’ G SOTO-ARES,’ J M CUISSET,’ J P NUYTS,’ L VALLiE’ ‘Services des Maladies lnfectieuses et Neurologie Infantiles; 2Service de Neuroradiologie, CHU de Lille, Lille, France Myelinolysis has rarely been reported in children. We report on a case devoid of central pontine myelinolysis (CPM). This boy presented at 3.5 years with a voluminous craniopharyngioma. Postoperatively, he developed diabetes insipidus, then a syndrome of inappropriate antidiuretic hormone. Hyponatremia was rapidly corrected with restriction of fluid (124 mEq/litre at day 7 to 140mEq/litre at day 8). On day 9 endocrine investigations were normal and the boy was sent home. On day 12, he developed cerebellar ataxia and ophthalmoplegia. Brain CT scan (day 20) and MRI (day 22) revealed isolated extrapontine myelinolysis (EPM). Myelinolysis may follow rapid correction of hyponatraemia, regardless of its cause. Pathology shows loss of myelin located in the central part of the basis pontis. In EPM, which accompanies 10% of patients, similar demyelinating lesions occur outside the pons with predilection for the basal ganglia. The neurologic syndrome typically ensues 2-32 days after hyponatraemia is corrected. In children, CPM and EPM have been reported in quite a number of diseases: adrenocortical insufficiency, leukaemia, diabetic ketoacidosis, and craniopharyngioma. CT scanning shows central pontine and extrapontine lesions as bilateral, symmetrical areas of hypodensity. Magnetic resonance imaging is more sensitive; lesions appear hypointense on Tl weighted and hyperintense on T2 weighted images and fluidattenuated inversion recovery sequences. Both CT and MRI may fail to show pathologically demonstrated demyelinating lesions. This may account for cases of EPM without CPM, as in our case. Yet there has been a report of one case that presented only the change of EPM, with pathologic confirmation. The outcome varies widely: some patients die, and others recover completely. Most reports decline a correlation between the severity of the initial symptoms, the severity of the MRI findings and the clinical condition. The rate of correction of hyponatraemia should be kept below 12mmol/litre/ 24 hours.
156 DNA-ploidy
in medulloblastoma
J M FOCK, A DAM, W M MOLENAAR Academic Hospital Groningen, Department of Neurology and Pathology, Groningen, The Netherlands Various clinical parameters are used to predict outcome of patients with medulloblastoma. Tumour staging and extent of surgical resection are the most important of
these. Still tumour behaviour is unpredictable and the search for additional objective methods for characterizing the tumour is ongoing. Flow cytometric DNA studies are known in solid tumours to be related to outcome. A few studies are done for medulloblastoma and aneuploidy is mostly correlated with good outcome. We looked at DNA-ploidy in our group of patients and compared the results with those in the literature. Between 1985 and 1998 39 patients younger than 16 years of age were treated in our institute with standard treatment: maximal resection of tumour mass, irradiation therapy and chemotherapy according to POG protocols. In 30 patients DNA flow cytometry was done successfully, two had only a biopsy and insufficient material for doing the technique; one patient had only cerebral fluid cytology and six samples will be repeated. In the 30 patients with clear histograms, 17 had a total or gross total resection, 11 a subtotal resection and two a biopsy. In the gross total/total resection group eight had a diploid tumour, two of them died (25%). In eight patients the tumour was aneuploid, three of them died (37%). Only one patient had a tetraploid tumour and is still surviving. In the subtotal resection group six patients had a diploid tumour; two died (33%), four tumours were aneuploid; these four patients died (100%) and one patient had a tetraploid tumour and died. The two tumour biopsies were aneuploid and both children died. Although the numbers are low, there seems to be a relation between ploidy and outcome. However, unlike most authors in the literature we found aneuploidy of poor outcome in cases with a subtotal resection.
102 Survey of parents/guardians or spinal cord tumours
of children with brain
K FREEMAN, C O’DELL, C MEOLA MONTEFIORE Medical Center, Bronx, New York, USA Purpose: The purpose was to identify needs of parents/ guardians of children with brain or spinal cord tumours at diagnosis, hospitalization, post-hospitalization, adjuvant treatment, recurrence, terminal or reported cured stage.
Methods: Survey instruments
for parents/guardians were tailored to stage of illness and the issues listed were derived from four focus groups involving 24 adults. Recruitment of survey participants was through health care providers, community organizations, or via the internet through listservers or web sites. Content of issues listed on survey instruments for each stage fell into four domains: interactions with health care providers, medical information and education, health service utilization and entitlements, and psychosocial issues. For each issue, the respondent was asked to indicate whether it was a problem and how important the issue was to them. Key issues reflect those with high prevalence for both the problem and importance determinations. Numbers in parentheses below indicate the prevalence of such issues.
Al 55
Abstracts
Results: Among 68 parents/guardians respondents, key issues at time of diagnosis included information about what caused the tumour (51%) and about treatment options (35%), and the manner the doctor conveyed the prognosis (36%). During the hospitalization, key issues concerned information about the prognosis (36%), results of surgery (29%), and impact on normal growth and development (31%). After hospital discharge, key issues were information about long-term effects/disabilities (33%) and the ability to care for the other children (26%). During times of adjuvant treatment, the key issue was the manner in which the doctor conveyed the prognosis (31%). During recurrence, the key issues were information about treatment options (32%) and the manner in which the doctor conveyed the prognosis (38%). For parents whose children were doing well, the key issue was information on possible long-term/ delayed side-effects of treatment (32%). During the last stage of life, key issues focused on information concerning treatment including risks and benefits (27%), stopping (30%), and for pain (33%), as well as preparing the family for the death (44%). Conclusions: More than half of families surveyed expressed that information they received regarding the cause of the tumour was a problem. Other problem areas include information regarding treatment, short- and long-term effects of treatment, the manner in which the prognosis was communicated, and preparing the family for the death of the child.
185 French adaptation of the health utilities index (HUI): A health-related quality of life instrument, in children with brain cancer C LE GALiS,’ N COSTET,’ j C GENTET,’ C KALIFA,3 D FRAPPAZ,4 C EDAN, E SARIBAN, D PLANTAZ,’ F DOZ’ ‘INSERM U357, Le Kremlin-Bicgtre, France; 2CHU La Timone, Marseille, France; 31nstitut Gustave Roussy, Villejuif, France; 4Centre L&on Bkard, Lyon, France; ‘CHR Sud, Rennes, France; 6Queen Fabiola Children’s Hospital, Brussels, Belgium; 7CHU de Grenoble, France; 81nstitut Curie, Paris, France Purpose: The objective of the study was to adapt and validate a health-related quality of life (HRQL) instrument, the HUI2-HU13 self-reported questionnaire, for children with brain cancer. The HUI was chosen because first, no HRQL instrument was available for children with cancer in the French context; second the HUI was originally developed in this paediatric population and finally, the development of a paediatric version was part of the global cross-cultural adaptation of the HUI in France. The adaptation of the HUI for children with cancer raised three issues: 1) the acceptability of the paediatric questionnaire by children with cancer; 2) the assessment of its psychometric properties; 3) the interrater agreement between the children, their parents and the physicians.
Materials and methods: The population was a group of 44 children with medulloblastoma already included in a multicentric database designed by the Group on Brain
Tumours in Children of the French Society for Paediatric Oncology to evaluate survival rates and sequellae associated with a new therapeutic regimen. The children were recruited during a routine consultation. Most of them had completed the treatment. The French adult questionnaire was linguistically adapted for children and tested by paediatric nurses, physicians and children. There were open-ended questions about the comprehensiveness of the questions to children. The main psychometric properties of the HU12 and HU13 classification systems were assessed in each group of raters (child, parent, physician): convergent and discriminant validities, internal consistency. Weighted and unweighted Kappa were used to measure the interrater agreement between the child’s parents’s and physician’s assessment of the child’s health state. Results: The use of HUI, in the mode in which it was tested (a self-report questionnaire for children, parents and physicians), proved itself to be easy to integrate into routine clinical activity. The face validity of the HUI questionnaire was favourably assessed. Criterion validity and internal consistency are good, especially when the physicians replied to the questionnaire. For the two other assessor groups, these are less clearly established, but any judgement should take into account the smaller number of participants on whom the statistics are based. The interpretability of the results obtained is sufficient, yet highlights the need of having a child population drawn from the general population, in order to measure better morbidity and the intensity of attribute deficits brought about by cancer. Nevertheless, the information gathered, especially relating to pain, emotion and cognition dimensions is particularly interesting. It confirms results recorded elsewhere concerning children suffering from brain cancer. Similarly, the results obtained relating to inter-rater reliability support the importance of the plurality of assessments, and especially the self-evaluation of HRQL, when the HUI is largely based on the evaluation of an individual‘s functional capacities.
173 Signs and/or symptoms leading to discovery or predicting progression of subependymal giant cell astrocytoma in children with tuberous sclerosis M RUGGIERI,’ A DISTEFANO,’ G PER0,3 L PAVONE’
G INCORPORA,’
‘Division of Paediatric Neurology; 31nstitute of Neuroradiology, University of Catania; ‘BFSNC, Catania, Italy
CNR,
Subependymal giant cell astrocytoma (SGCA) are histologically benign lesions that typically arise from subependymal nodules in the anterior lateral ventricles in 6-14% of tuberous sclerosis complex patients. They are usually not aggressively invasive, and surgery can be curative if done early. Thus, early identification on a SGCA, by any means, could decrease the operative morbidity and improve the outcome. Several radiological studies tried to identify neuroimaging criteria for early diagnosis of SGCA. Background:
Abstracts
Section XIX: Turnout-s 281 Isolated extrapontine myelinolysis for craniopharyngioma
following
surgery
J C CUVELLIER,’ C DE SiZE,’ G SOTO-ARES,’ J M CUISSET,’ J P NUYTS,’ L VALLiE’ ‘Services des Maladies lnfectieuses et Neurologie Infantiles; 2Service de Neuroradiologie, CHU de Lille, Lille, France Myelinolysis has rarely been reported in children. We report on a case devoid of central pontine myelinolysis (CPM). This boy presented at 3.5 years with a voluminous craniopharyngioma. Postoperatively, he developed diabetes insipidus, then a syndrome of inappropriate antidiuretic hormone. Hyponatremia was rapidly corrected with restriction of fluid (124 mEq/litre at day 7 to 140mEq/litre at day 8). On day 9 endocrine investigations were normal and the boy was sent home. On day 12, he developed cerebellar ataxia and ophthalmoplegia. Brain CT scan (day 20) and MRI (day 22) revealed isolated extrapontine myelinolysis (EPM). Myelinolysis may follow rapid correction of hyponatraemia, regardless of its cause. Pathology shows loss of myelin located in the central part of the basis pontis. In EPM, which accompanies 10% of patients, similar demyelinating lesions occur outside the pons with predilection for the basal ganglia. The neurologic syndrome typically ensues 2-32 days after hyponatraemia is corrected. In children, CPM and EPM have been reported in quite a number of diseases: adrenocortical insufficiency, leukaemia, diabetic ketoacidosis, and craniopharyngioma. CT scanning shows central pontine and extrapontine lesions as bilateral, symmetrical areas of hypodensity. Magnetic resonance imaging is more sensitive; lesions appear hypointense on Tl weighted and hyperintense on T2 weighted images and fluidattenuated inversion recovery sequences. Both CT and MRI may fail to show pathologically demonstrated demyelinating lesions. This may account for cases of EPM without CPM, as in our case. Yet there has been a report of one case that presented only the change of EPM, with pathologic confirmation. The outcome varies widely: some patients die, and others recover completely. Most reports decline a correlation between the severity of the initial symptoms, the severity of the MRI findings and the clinical condition. The rate of correction of hyponatraemia should be kept below 12mmol/litre/ 24 hours.
156 DNA-ploidy
in medulloblastoma
J M FOCK, A DAM, W M MOLENAAR Academic Hospital Groningen, Department of Neurology and Pathology, Groningen, The Netherlands Various clinical parameters are used to predict outcome of patients with medulloblastoma. Tumour staging and extent of surgical resection are the most important of
these. Still tumour behaviour is unpredictable and the search for additional objective methods for characterizing the tumour is ongoing. Flow cytometric DNA studies are known in solid tumours to be related to outcome. A few studies are done for medulloblastoma and aneuploidy is mostly correlated with good outcome. We looked at DNA-ploidy in our group of patients and compared the results with those in the literature. Between 1985 and 1998 39 patients younger than 16 years of age were treated in our institute with standard treatment: maximal resection of tumour mass, irradiation therapy and chemotherapy according to POG protocols. In 30 patients DNA flow cytometry was done successfully, two had only a biopsy and insufficient material for doing the technique; one patient had only cerebral fluid cytology and six samples will be repeated. In the 30 patients with clear histograms, 17 had a total or gross total resection, 11 a subtotal resection and two a biopsy. In the gross total/total resection group eight had a diploid tumour, two of them died (25%). In eight patients the tumour was aneuploid, three of them died (37%). Only one patient had a tetraploid tumour and is still surviving. In the subtotal resection group six patients had a diploid tumour; two died (33%), four tumours were aneuploid; these four patients died (100%) and one patient had a tetraploid tumour and died. The two tumour biopsies were aneuploid and both children died. Although the numbers are low, there seems to be a relation between ploidy and outcome. However, unlike most authors in the literature we found aneuploidy of poor outcome in cases with a subtotal resection.
102 Survey of parents/guardians or spinal cord tumours
of children with brain
K FREEMAN, C O’DELL, C MEOLA MONTEFIORE Medical Center, Bronx, New York, USA Purpose: The purpose was to identify needs of parents/ guardians of children with brain or spinal cord tumours at diagnosis, hospitalization, post-hospitalization, adjuvant treatment, recurrence, terminal or reported cured stage.
Methods: Survey instruments
for parents/guardians were tailored to stage of illness and the issues listed were derived from four focus groups involving 24 adults. Recruitment of survey participants was through health care providers, community organizations, or via the internet through listservers or web sites. Content of issues listed on survey instruments for each stage fell into four domains: interactions with health care providers, medical information and education, health service utilization and entitlements, and psychosocial issues. For each issue, the respondent was asked to indicate whether it was a problem and how important the issue was to them. Key issues reflect those with high prevalence for both the problem and importance determinations. Numbers in parentheses below indicate the prevalence of such issues.
Al 55
Abstracts
Results: Among 68 parents/guardians respondents, key issues at time of diagnosis included information about what caused the tumour (51%) and about treatment options (35%), and the manner the doctor conveyed the prognosis (36%). During the hospitalization, key issues concerned information about the prognosis (36%), results of surgery (29%), and impact on normal growth and development (31%). After hospital discharge, key issues were information about long-term effects/disabilities (33%) and the ability to care for the other children (26%). During times of adjuvant treatment, the key issue was the manner in which the doctor conveyed the prognosis (31%). During recurrence, the key issues were information about treatment options (32%) and the manner in which the doctor conveyed the prognosis (38%). For parents whose children were doing well, the key issue was information on possible long-term/ delayed side-effects of treatment (32%). During the last stage of life, key issues focused on information concerning treatment including risks and benefits (27%), stopping (30%), and for pain (33%), as well as preparing the family for the death (44%). Conclusions: More than half of families surveyed expressed that information they received regarding the cause of the tumour was a problem. Other problem areas include information regarding treatment, short- and long-term effects of treatment, the manner in which the prognosis was communicated, and preparing the family for the death of the child.
185 French adaptation of the health utilities index (HUI): A health-related quality of life instrument, in children with brain cancer C LE GALiS,’ N COSTET,’ j C GENTET,’ C KALIFA,3 D FRAPPAZ,4 C EDAN, E SARIBAN, D PLANTAZ,’ F DOZ’ ‘INSERM U357, Le Kremlin-Bicgtre, France; 2CHU La Timone, Marseille, France; 31nstitut Gustave Roussy, Villejuif, France; 4Centre L&on Bkard, Lyon, France; ‘CHR Sud, Rennes, France; 6Queen Fabiola Children’s Hospital, Brussels, Belgium; 7CHU de Grenoble, France; 81nstitut Curie, Paris, France Purpose: The objective of the study was to adapt and validate a health-related quality of life (HRQL) instrument, the HUI2-HU13 self-reported questionnaire, for children with brain cancer. The HUI was chosen because first, no HRQL instrument was available for children with cancer in the French context; second the HUI was originally developed in this paediatric population and finally, the development of a paediatric version was part of the global cross-cultural adaptation of the HUI in France. The adaptation of the HUI for children with cancer raised three issues: 1) the acceptability of the paediatric questionnaire by children with cancer; 2) the assessment of its psychometric properties; 3) the interrater agreement between the children, their parents and the physicians.
Materials and methods: The population was a group of 44 children with medulloblastoma already included in a multicentric database designed by the Group on Brain
Tumours in Children of the French Society for Paediatric Oncology to evaluate survival rates and sequellae associated with a new therapeutic regimen. The children were recruited during a routine consultation. Most of them had completed the treatment. The French adult questionnaire was linguistically adapted for children and tested by paediatric nurses, physicians and children. There were open-ended questions about the comprehensiveness of the questions to children. The main psychometric properties of the HU12 and HU13 classification systems were assessed in each group of raters (child, parent, physician): convergent and discriminant validities, internal consistency. Weighted and unweighted Kappa were used to measure the interrater agreement between the child’s parents’s and physician’s assessment of the child’s health state. Results: The use of HUI, in the mode in which it was tested (a self-report questionnaire for children, parents and physicians), proved itself to be easy to integrate into routine clinical activity. The face validity of the HUI questionnaire was favourably assessed. Criterion validity and internal consistency are good, especially when the physicians replied to the questionnaire. For the two other assessor groups, these are less clearly established, but any judgement should take into account the smaller number of participants on whom the statistics are based. The interpretability of the results obtained is sufficient, yet highlights the need of having a child population drawn from the general population, in order to measure better morbidity and the intensity of attribute deficits brought about by cancer. Nevertheless, the information gathered, especially relating to pain, emotion and cognition dimensions is particularly interesting. It confirms results recorded elsewhere concerning children suffering from brain cancer. Similarly, the results obtained relating to inter-rater reliability support the importance of the plurality of assessments, and especially the self-evaluation of HRQL, when the HUI is largely based on the evaluation of an individual‘s functional capacities.
173 Signs and/or symptoms leading to discovery or predicting progression of subependymal giant cell astrocytoma in children with tuberous sclerosis M RUGGIERI,’ A DISTEFANO,’ G PER0,3 L PAVONE’
G INCORPORA,’
‘Division of Paediatric Neurology; 31nstitute of Neuroradiology, University of Catania; ‘BFSNC, Catania, Italy
CNR,
Subependymal giant cell astrocytoma (SGCA) are histologically benign lesions that typically arise from subependymal nodules in the anterior lateral ventricles in 6-14% of tuberous sclerosis complex patients. They are usually not aggressively invasive, and surgery can be curative if done early. Thus, early identification on a SGCA, by any means, could decrease the operative morbidity and improve the outcome. Several radiological studies tried to identify neuroimaging criteria for early diagnosis of SGCA. Background: